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Lymphadenitis

MedGen UID:
7410
Concept ID:
C0024205
Disease or Syndrome
Synonym: Lymphadenitis (disease)
SNOMED CT: Lymphadenitis (19471005); Adenitis (19471005); Inflammation of lymph node (19471005)
 
HPO: HP:0002840
Monarch Initiative: MONDO:0002052

Definition

Inflammation of a lymph node. [from HPO]

Conditions with this feature

Hyperimmunoglobulin D with periodic fever
MedGen UID:
140768
Concept ID:
C0398691
Disease or Syndrome
Mevalonate kinase deficiency is a condition characterized by recurrent episodes of fever, which typically begin during infancy. Each episode of fever lasts about 3 to 6 days, and the frequency of the episodes varies among affected individuals. In childhood the fevers seem to be more frequent, occurring as often as 25 times a year, but as the individual gets older the episodes occur less often.\n\nMevalonate kinase deficiency has additional signs and symptoms, and the severity depends on the type of the condition. There are two types of mevalonate kinase deficiency: a less severe type called hyperimmunoglobulinemia D syndrome (HIDS) and a more severe type called mevalonic aciduria (MVA).\n\nDuring episodes of fever, people with HIDS typically have enlargement of the lymph nodes (lymphadenopathy), abdominal pain, joint pain, diarrhea, skin rashes, and headache. Occasionally they will have painful sores called aphthous ulcers around their mouth. In females, these may also occur around the vagina. Rarely, people with HIDS develop a buildup of protein deposits (amyloidosis) in the kidneys that can lead to kidney failure. Fever episodes in individuals with HIDS can be triggered by vaccinations, surgery, injury, or stress. Most people with HIDS have abnormally high levels of immune system proteins called immunoglobulin D (IgD) and immunoglobulin A (IgA) in the blood. It is unclear why some people with HIDS have high levels of IgD and IgA and some do not. Elevated levels of these immunoglobulins do not appear to cause any signs or symptoms. Individuals with HIDS do not have any signs and symptoms of the condition between fever episodes and typically have a normal life expectancy.\n\nPeople with MVA have signs and symptoms of the condition at all times, not just during episodes of fever. Affected children have developmental delay, problems with movement and balance (ataxia), recurrent seizures (epilepsy), progressive problems with vision, and failure to gain weight and grow at the expected rate (failure to thrive). Individuals with MVA typically have an unusually small, elongated head. In childhood or adolescence, affected individuals may develop eye problems such as inflammation of the eye (uveitis), a blue tint in the white part of the eye (blue sclera), an eye disorder called retinitis pigmentosa that causes vision loss, or clouding of the lens of the eye (cataracts). Affected adults may have short stature and may develop muscle weakness (myopathy) later in life. During fever episodes, people with MVA may have an enlarged liver and spleen (hepatosplenomegaly), lymphadenopathy, abdominal pain, diarrhea, and skin rashes. Children with MVA who are severely affected with multiple problems may live only into early childhood; mildly affected individuals may have a normal life expectancy.
Granulomatous disease, chronic, X-linked
MedGen UID:
336165
Concept ID:
C1844376
Disease or Syndrome
Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder of phagocytes (neutrophils, monocytes, macrophages, and eosinophils) resulting from impaired killing of bacteria and fungi. CGD is characterized by severe recurrent bacterial and fungal infections and dysregulated inflammatory responses resulting in granuloma formation and other inflammatory disorders such as colitis. Infections typically involve the lung (pneumonia), lymph nodes (lymphadenitis), liver (abscess), bone (osteomyelitis), and skin (abscesses or cellulitis). Granulomas typically involve the genitourinary system (bladder) and gastrointestinal tract (often the pylorus initially, and later the esophagus, jejunum, ileum, cecum, rectum, and perirectal area). Some males with X-linked CGD have McLeod neuroacanthocytosis syndrome as the result of a contiguous gene deletion. While CGD may present anytime from infancy to late adulthood, the vast majority of affected individuals are diagnosed before age five years. Use of antimicrobial prophylaxis and therapy has greatly improved overall survival.
Granulomatous disease, chronic, autosomal recessive, cytochrome b-positive, type 2
MedGen UID:
383869
Concept ID:
C1856245
Disease or Syndrome
Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder of phagocytes (neutrophils, monocytes, macrophages, and eosinophils) resulting from impaired killing of bacteria and fungi. CGD is characterized by severe recurrent bacterial and fungal infections and dysregulated inflammatory responses resulting in granuloma formation and other inflammatory disorders such as colitis. Infections typically involve the lung (pneumonia), lymph nodes (lymphadenitis), liver (abscess), bone (osteomyelitis), and skin (abscesses or cellulitis). Granulomas typically involve the genitourinary system (bladder) and gastrointestinal tract (often the pylorus initially, and later the esophagus, jejunum, ileum, cecum, rectum, and perirectal area). Some males with X-linked CGD have McLeod neuroacanthocytosis syndrome as the result of a contiguous gene deletion. While CGD may present anytime from infancy to late adulthood, the vast majority of affected individuals are diagnosed before age five years. Use of antimicrobial prophylaxis and therapy has greatly improved overall survival.
Granulomatous disease, chronic, autosomal recessive, cytochrome b-positive, type 1
MedGen UID:
341102
Concept ID:
C1856251
Disease or Syndrome
Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder of phagocytes (neutrophils, monocytes, macrophages, and eosinophils) resulting from impaired killing of bacteria and fungi. CGD is characterized by severe recurrent bacterial and fungal infections and dysregulated inflammatory responses resulting in granuloma formation and other inflammatory disorders such as colitis. Infections typically involve the lung (pneumonia), lymph nodes (lymphadenitis), liver (abscess), bone (osteomyelitis), and skin (abscesses or cellulitis). Granulomas typically involve the genitourinary system (bladder) and gastrointestinal tract (often the pylorus initially, and later the esophagus, jejunum, ileum, cecum, rectum, and perirectal area). Some males with X-linked CGD have McLeod neuroacanthocytosis syndrome as the result of a contiguous gene deletion. While CGD may present anytime from infancy to late adulthood, the vast majority of affected individuals are diagnosed before age five years. Use of antimicrobial prophylaxis and therapy has greatly improved overall survival.
Granulomatous disease, chronic, autosomal recessive, cytochrome b-negative
MedGen UID:
383872
Concept ID:
C1856255
Disease or Syndrome
Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder of phagocytes (neutrophils, monocytes, macrophages, and eosinophils) resulting from impaired killing of bacteria and fungi. CGD is characterized by severe recurrent bacterial and fungal infections and dysregulated inflammatory responses resulting in granuloma formation and other inflammatory disorders such as colitis. Infections typically involve the lung (pneumonia), lymph nodes (lymphadenitis), liver (abscess), bone (osteomyelitis), and skin (abscesses or cellulitis). Granulomas typically involve the genitourinary system (bladder) and gastrointestinal tract (often the pylorus initially, and later the esophagus, jejunum, ileum, cecum, rectum, and perirectal area). Some males with X-linked CGD have McLeod neuroacanthocytosis syndrome as the result of a contiguous gene deletion. While CGD may present anytime from infancy to late adulthood, the vast majority of affected individuals are diagnosed before age five years. Use of antimicrobial prophylaxis and therapy has greatly improved overall survival.
Pyogenic bacterial infections due to MyD88 deficiency
MedGen UID:
383023
Concept ID:
C2677092
Disease or Syndrome
Immunodeficiency-68 (IMD68) is an autosomal recessive primary immunodeficiency characterized by severe systemic and invasive bacterial infections beginning in infancy or early childhood. The most common organisms implicated are Streptococcus pneumoniae, Staphylococcus aureus, and Pseudomonas, although other organisms may be observed. IMD68 is life-threatening in infancy and early childhood. The first invasive infection typically occurs before 2 years of age, with meningitis and upper respiratory infections being common manifestations. The mortality rate in early childhood is high, with most deaths occurring before 8 years of age. Affected individuals have an impaired inflammatory response to infection, including lack of fever and neutropenia, although erythrocyte sedimentation rate (ESR) and C-reactive protein may be elevated. General immunologic workup tends to be normal, with normal levels of B cells, T cells, and NK cells. However, more detailed studies indicate impaired cytokine response to lipopolysaccharide (LPS) and IL1B (147720) stimulation; response to TNFA (191160) is usually normal. Patients have good antibody responses to most vaccinations. Viral, fungal, and parasitic infections are generally not observed. Early detection is critical in early childhood because prophylactic treatment with IVIg or certain antibiotics is effective; the disorder tends to improve naturally around adolescence. At the molecular level, IMD68 results from impaired function of selective Toll receptor (see TLR4, 603030)/IL1R (see IL1R1; 147810) signaling pathways that ultimately activate NFKB (164011) to produce cytokines (summary by Picard et al., 2010). See also IMD67 (607676), caused by mutation in the IRAK4 gene (602170), which shows a similar phenotype to IMD68. As the MYD88 and IRAK4 genes interact in the same intracellular signaling pathway, the clinical and cellular features are almost indistinguishable (summary by Picard et al., 2010).
Mendelian susceptibility to mycobacterial diseases due to partial IRF8 deficiency
MedGen UID:
814919
Concept ID:
C3808589
Disease or Syndrome
Autosomal dominant IRF8 deficiency, or IMD32A, causes an abnormal peripheral blood myeloid phenotype with a marked loss of CD11C (ITGAX; 151510)-positive/CD1C (188340)-positive dendritic cells, resulting in selective susceptibility to mycobacterial infections (Hambleton et al., 2011).
Polyglucosan body myopathy type 1
MedGen UID:
863042
Concept ID:
C4014605
Disease or Syndrome
Polyglucosan body myopathy-1 (PGBM1) is an autosomal recessive disorder characterized by onset in childhood of progressive proximal muscle weakness, resulting in difficulties in ambulation. Most patients also develop progressive dilated cardiomyopathy, which may necessitate cardiac transplant in severe cases. A small subset of patients present with severe immunodeficiency and a hyperinflammatory state in very early childhood (summary by Boisson et al., 2012 and Nilsson et al., 2013). Genetic Heterogeneity of Polyglucosan Body Myopathy See also PGBM2 (616199), caused by mutation in the GYG1 gene (603942) on chromosome 3q24.
Pseudo-TORCH syndrome 3
MedGen UID:
1708513
Concept ID:
C5394391
Disease or Syndrome
Pseudo-TORCH syndrome-3 (PTORCH3) is an autosomal recessive disorder of immune dysregulation and neuroinflammation apparent from early infancy. Affected individuals have developmental delay with acute episodes of fever and multisystemic organ involvement, including coagulopathy, elevated liver enzymes, and proteinuria, often associated with thrombotic microangiopathy. Brain imaging shows progressive intracranial calcifications, white matter abnormalities, and sometimes cerebral or cerebellar atrophy. Laboratory studies show abnormal elevation of interferon (IFN)-stimulated gene (ISG) transcripts consistent with a type I interferonopathy. The phenotype resembles the sequelae of intrauterine infection, but there is usually no evidence of an infectious agent. The disorder results from defects in negative regulation of the interferon immunologic pathway. Death in early childhood is common (summary by Duncan et al., 2019 and Gruber et al., 2020). For a discussion of genetic heterogeneity of PTORCH, see PTORCH1 (251290).
Granulomatous disease, chronic, autosomal recessive, 5
MedGen UID:
1710326
Concept ID:
C5394542
Disease or Syndrome
Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder of phagocytes (neutrophils, monocytes, macrophages, and eosinophils) resulting from impaired killing of bacteria and fungi. CGD is characterized by severe recurrent bacterial and fungal infections and dysregulated inflammatory responses resulting in granuloma formation and other inflammatory disorders such as colitis. Infections typically involve the lung (pneumonia), lymph nodes (lymphadenitis), liver (abscess), bone (osteomyelitis), and skin (abscesses or cellulitis). Granulomas typically involve the genitourinary system (bladder) and gastrointestinal tract (often the pylorus initially, and later the esophagus, jejunum, ileum, cecum, rectum, and perirectal area). Some males with X-linked CGD have McLeod neuroacanthocytosis syndrome as the result of a contiguous gene deletion. While CGD may present anytime from infancy to late adulthood, the vast majority of affected individuals are diagnosed before age five years. Use of antimicrobial prophylaxis and therapy has greatly improved overall survival.
Immunodeficiency 73b with defective neutrophil chemotaxis and lymphopenia
MedGen UID:
1740566
Concept ID:
C5436549
Disease or Syndrome
Immunodeficiency-73B with defective neutrophil chemotaxis (IMD73B) is an autosomal dominant immunologic disorder characterized by onset of recurrent infections in infancy or early childhood. Affected individuals develop respiratory infections, cellulitis, and severe invasive infections or sepsis; organisms include bacteria such as Staphylococcus, as well as viruses, fungi, and mycobacterial species. Laboratory studies show variable abnormalities, including B- and T-cell lymphopenia, decreased immunoglobulin subsets, decreased TRECs and dysfunctional T cells, decreased NK cells, neutropenia, and impaired neutrophil chemotaxis. Hematopoietic stem cell transplantation is curative (summary by Hsu et al., 2019; review by Lougaris et al., 2020). In a review of autosomal forms of chronic granulomatous disease (see 306400 for genetic heterogeneity of CGD), Roos et al. (2021) noted that patients with RAC2 mutations may manifest CGD-like symptoms due to defects in neutrophil NADPH oxidase activity.

Professional guidelines

PubMed

Roy CF, Balakrishnan K, Boudewyns A, Cheng A, Chun RH, Daniel SJ, Fayoux P, Hart C, Hemansson A, Hewitt R, Hsu WC, Kuo M, Liu C, Maddalozzo J, Messner AH, Pransky S, Rahbar R, Rickert S, Roy S, Russell J, Rutter MJ, Sie KCY, Sidell D, Smith R, Soma M, Spratley J, Watters K, White DR, Wolter N, Zalzal G, Yeung JC
Int J Pediatr Otorhinolaryngol 2023 Mar;166:111469. Epub 2023 Feb 2 doi: 10.1016/j.ijporl.2023.111469. PMID: 36764081
Boitsaniuk SI, Levkiv MО, Fedoniuk LY, Kuzniak NB, Bambuliak AV
Wiad Lek 2022;75(1 pt 2):318-323. PMID: 35182142
Gaddey HL, Riegel AM
Am Fam Physician 2016 Dec 1;94(11):896-903. PMID: 27929264

Recent clinical studies

Etiology

Roy CF, Balakrishnan K, Boudewyns A, Cheng A, Chun RH, Daniel SJ, Fayoux P, Hart C, Hemansson A, Hewitt R, Hsu WC, Kuo M, Liu C, Maddalozzo J, Messner AH, Pransky S, Rahbar R, Rickert S, Roy S, Russell J, Rutter MJ, Sie KCY, Sidell D, Smith R, Soma M, Spratley J, Watters K, White DR, Wolter N, Zalzal G, Yeung JC
Int J Pediatr Otorhinolaryngol 2023 Mar;166:111469. Epub 2023 Feb 2 doi: 10.1016/j.ijporl.2023.111469. PMID: 36764081
Zenebe Y, Adem Y, Tulu B, Mekonnen D, Derbie A, Mekonnen Z, Biadglegne F
Ethiop J Health Sci 2021 May;31(3):653-662. doi: 10.4314/ejhs.v31i3.23. PMID: 34483623Free PMC Article
Nair IR, Balan S, Phalak P, Daniel M
Indian J Pathol Microbiol 2020 Jan-Mar;63(1):60-63. doi: 10.4103/IJPM.IJPM_622_19. PMID: 32031124
Rosenberg TL, Nolder AR
Otolaryngol Clin North Am 2014 Oct;47(5):721-31. Epub 2014 Jul 30 doi: 10.1016/j.otc.2014.06.012. PMID: 25213279
Leung AK, Robson WL
J Pediatr Health Care 2004 Jan-Feb;18(1):3-7. doi: 10.1016/j.pedhc.2003.08.008. PMID: 14722499Free PMC Article

Diagnosis

Faraz M, Rosado FGN
Clin Lab Med 2021 Sep;41(3):433-451. Epub 2021 Jul 2 doi: 10.1016/j.cll.2021.04.001. PMID: 34304774
Alnæs M, Mjelle K, Vintermyr O
Tidsskr Nor Laegeforen 2020 Jan 14;140(1) Epub 2020 Jan 6 doi: 10.4045/tidsskr.19.0500. PMID: 31948199
Perry AM, Choi SM
Arch Pathol Lab Med 2018 Nov;142(11):1341-1346. doi: 10.5858/arpa.2018-0219-RA. PMID: 30407860
Alam H, Saeed MO, Saeed AB, Zaidi A
J Pak Med Assoc 2015 Dec;65(12):1349-50. PMID: 26627522
Hutchinson CB, Wang E
Arch Pathol Lab Med 2010 Feb;134(2):289-93. doi: 10.5858/134.2.289. PMID: 20121621

Therapy

Roy CF, Balakrishnan K, Boudewyns A, Cheng A, Chun RH, Daniel SJ, Fayoux P, Hart C, Hemansson A, Hewitt R, Hsu WC, Kuo M, Liu C, Maddalozzo J, Messner AH, Pransky S, Rahbar R, Rickert S, Roy S, Russell J, Rutter MJ, Sie KCY, Sidell D, Smith R, Soma M, Spratley J, Watters K, White DR, Wolter N, Zalzal G, Yeung JC
Int J Pediatr Otorhinolaryngol 2023 Mar;166:111469. Epub 2023 Feb 2 doi: 10.1016/j.ijporl.2023.111469. PMID: 36764081
Lin HS, Lin PT, Tsai YS, Wang SH, Chi CC
Cochrane Database Syst Rev 2021 Feb 26;2(2):CD013099. doi: 10.1002/14651858.CD013099.pub2. PMID: 33634465Free PMC Article
Soriano A, Soriano M, Espinosa G, Manna R, Emmi G, Cantarini L, Hernández-Rodríguez J
Front Immunol 2020;11:865. Epub 2020 Jun 3 doi: 10.3389/fimmu.2020.00865. PMID: 32655539Free PMC Article
McClay JE, Garcia C
Curr Opin Otolaryngol Head Neck Surg 2013 Dec;21(6):581-7. doi: 10.1097/MOO.0000000000000005. PMID: 24152916
Goraya JS, Virdi VS
Postgrad Med J 2002 Jun;78(920):327-9. doi: 10.1136/pmj.78.920.327. PMID: 12151684Free PMC Article

Prognosis

Nair IR, Balan S, Phalak P, Daniel M
Indian J Pathol Microbiol 2020 Jan-Mar;63(1):60-63. doi: 10.4103/IJPM.IJPM_622_19. PMID: 32031124
Pepe F, Disma S, Teodoro C, Pepe P, Magro G
Pathologica 2016 Sep;108(3):120-129. PMID: 28195263
Rosenberg TL, Nolder AR
Otolaryngol Clin North Am 2014 Oct;47(5):721-31. Epub 2014 Jul 30 doi: 10.1016/j.otc.2014.06.012. PMID: 25213279
Biadglegne F, Tesfaye W, Anagaw B, Tessema B, Debebe T, Anagaw B, Mulu A, Sack U, Rodloff AC
Jpn J Infect Dis 2013;66(4):263-8. doi: 10.7883/yoken.66.263. PMID: 23883834
Onciu M, Medeiros LJ
Adv Anat Pathol 2003 Jul;10(4):204-11. doi: 10.1097/00125480-200307000-00003. PMID: 12826826

Clinical prediction guides

Manthiram K
Curr Opin Rheumatol 2023 Nov 1;35(6):423-428. Epub 2023 Jul 17 doi: 10.1097/BOR.0000000000000956. PMID: 37467064Free PMC Article
Roy CF, Balakrishnan K, Boudewyns A, Cheng A, Chun RH, Daniel SJ, Fayoux P, Hart C, Hemansson A, Hewitt R, Hsu WC, Kuo M, Liu C, Maddalozzo J, Messner AH, Pransky S, Rahbar R, Rickert S, Roy S, Russell J, Rutter MJ, Sie KCY, Sidell D, Smith R, Soma M, Spratley J, Watters K, White DR, Wolter N, Zalzal G, Yeung JC
Int J Pediatr Otorhinolaryngol 2023 Mar;166:111469. Epub 2023 Feb 2 doi: 10.1016/j.ijporl.2023.111469. PMID: 36764081
Nair IR, Balan S, Phalak P, Daniel M
Indian J Pathol Microbiol 2020 Jan-Mar;63(1):60-63. doi: 10.4103/IJPM.IJPM_622_19. PMID: 32031124
Mahawerawat K, Kasemsiri P
J Laryngol Otol 2018 Sep;132(9):827-831. Epub 2018 Sep 5 doi: 10.1017/S0022215118001391. PMID: 30180912
Pepe F, Disma S, Teodoro C, Pepe P, Magro G
Pathologica 2016 Sep;108(3):120-129. PMID: 28195263

Recent systematic reviews

Fonnes S, Rasmussen T, Brunchmann A, Holzknecht BJ, Rosenberg J
J Surg Res 2022 Feb;270:12-21. Epub 2021 Oct 7 doi: 10.1016/j.jss.2021.08.027. PMID: 34628159
Villanueva P, Pittet LF, Curtis N
Pediatr Infect Dis J 2021 Nov 1;40(11):1037-1045. doi: 10.1097/INF.0000000000003237. PMID: 34636800
Lin HS, Lin PT, Tsai YS, Wang SH, Chi CC
Cochrane Database Syst Rev 2021 Feb 26;2(2):CD013099. doi: 10.1002/14651858.CD013099.pub2. PMID: 33634465Free PMC Article
Willemse SH, Oomens MAEM, De Lange J, Karssemakers LHE
Int J Pediatr Otorhinolaryngol 2018 Sep;112:48-54. Epub 2018 Jun 19 doi: 10.1016/j.ijporl.2018.06.034. PMID: 30055739
Quaglio G, Pizzol D, Bortolani A, Manenti F, Isaakidis P, Putoto G, Olliaro PL
PLoS One 2018;13(4):e0194766. Epub 2018 Apr 3 doi: 10.1371/journal.pone.0194766. PMID: 29614082Free PMC Article

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